Outcomes of Hypoplastic Left Heart Syndrome by Subtype and Presence of Ventriculocoronary Connections: A Fetal Heart Society Multicenter Retrospective Study.

MS/AA hypoplastic left heart syndrome with ventriculocoronary connections had the lowest transplant-free survival, with a hazard ratio of 1.69 versus MS/AA without VCCs.

• Why it matters: Subtype-specific outcomes in HLHS are clinically important, and VCCs may further stratify mortality risk beyond anatomic classification.
• What they did: In a multicenter retrospective cohort of 591 fetuses/infants with HLHS (born alive, intention to treat; 2012–2016), Cox models adjusted for center, birth weight, genetics, extracardiac malformations, and atrial septum status.
• The result: Overall TFS was 55.6% at 5 years and 49.4% at 10 years; MS/AA with VCCs had 44.7% (5-year) and 40.3% (10-year) TFS, and prenatal VCC suspicion was linked to lower TFS (HR 1.79).